FAMILIAL ERYTHROBLASTIC ANEMIA THALASSEMIA—COOLEY'S ANEMIA
- 8 May 1943
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA
- Vol. 122 (2) , 83-86
- https://doi.org/10.1001/jama.1943.02840190013005
Abstract
Since Cooley's1definite crystallization of a group of clinical findings as a disease entity, familial erythroblastic anemia has received worldwide recognition and confirmation. Cooley stressed a congenital cause, evidence of bone marrow stimulation, leukocytosis, absence of increased fragility of the red blood cells, splenomegaly, osteoporosis and a peculiar mongoloid facial appearance. As reports of cases appeared, the scope of investigation widened until it became clear that familial erythroblastic anemia was in reality a severe and usually fatal form, in childhood, of what is essentially a hereditary, chronic disease of Mediterranean races, characterized by splenomegaly and certain characteristic changes in the blood. In fact, this greatly widened concept of the disorder led Whipple and Bradford2to suggest the name "thalassemia" or "Mediterranean disease" rather than the term "erythroblastic anemia." Indeed in our opinion the latter term cannot help but become confused with fetal erythroblastosis. The use of the word anemiaKeywords
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